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Recurrent encephalopathy? No I'm a sleeping beauty!
Mehtab Iqbal1, Manish Prasad1, Christopher Ritey1
1Department of Neurology, Sheffield Children's Hospital, Sheffield, South Yorkshire, UK.
Journal of Pediatric Neurosciences
|June 4, 2014
Summary
Kleine-Levin syndrome (KLS), a rare disorder, presents in adolescent males with recurrent episodes of excessive sleepiness, cognitive and behavioral changes, and hypersexuality. This case highlights the diagnostic challenge of KLS, often mimicking encephalopathy with normal initial investigations.
Area of Science:
- Neurology
- Pediatrics
- Sleep Medicine
Background:
- Kleine-Levin syndrome (KLS), also known as Sleeping Beauty syndrome, is a rare neurological disorder characterized by recurrent episodes of hypersomnia and cognitive/behavioral disturbances.
- It primarily affects adolescent males and can significantly impact their quality of life and academic performance.
Observation:
- A 14-year-old boy presented with acute excessive tiredness, hypersomnia, abnormal behavior, and hypersexuality following a viral infection.
- Initial neurological and systemic examinations, including MRI brain scan and CSF analysis, were unremarkable.
- Electroencephalogram (EEG) revealed non-specific diffuse slowing consistent with encephalopathy.
Findings:
- The patient experienced recurrent episodes of hypersomnia, cognitive impairment, behavioral changes, and hypersexuality, consistent with KLS.
- Symptoms resolved spontaneously within approximately three weeks per episode.
- Recurrent episodes occurred despite normal initial investigations, with only EEG showing non-specific slowing.
Implications:
- This case underscores the diagnostic challenges in identifying KLS, often requiring a high index of suspicion due to its episodic nature and normal initial workup.
- Early recognition and management of KLS are crucial for affected adolescents, potentially involving stimulant use for somnolence.
- Further research into the pathophysiology and treatment of KLS is warranted to improve patient outcomes.
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